Table 1.
Clinical features, immunohistochemical findings, ANCA details, and CRP level.
| Case no. | Sex | Age at Dx | Biopsy site | IgG4 average | IgG4/IgG ratio | Path Dx score | Clinical Dx (modified ACR criteria) | ANCA (+) | P-ANCA | MPO | C-ANCA | PR3 | CRP |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | M | 74 | Nasal septum | 28 | 87% | 3 | Generalized | y | n | n | y | y | High |
| 2 | F | 83 | Nasal sinus | 20 | 63% | 3 | Limited | y | n | n | y | y | High |
| 3 | M | 54 | Nasal septum | 62 | 59% | 3 | Generalized | y | n | n | y | y | High |
| 4 | F | 59 | Lung RUL RML | 27 | 28% | 3 | Limited | y | y | y | n | n | NA |
| 5 | F | 19 | Oral cavity | 6 | 19% | 3 | Generalized | y | n | n | y | y | NA |
| 6 | F | 18 | Nasal sinus | 11 | 79% | 1 | Generalized | y | y | y | n | n | NA |
| 7* | M | 47 | Orbit | 49 | 58% | 2 | Generalized | y | n | n | y | y | High* |
| 8* | M | 47 | Nasal septum | 4 | 19% | 1 | Generalized | y | n | n | y | y | * |
| 9** | F | 31 | Lung, left, pneumonectomy | 16 | 33% | 3 | Generalized | y | n | n | y | y | NA** |
| 10** | F | 31 | Kidney | 26 | 39% | 3 | Generalized | y | n | n | y | y | ** |
| 11 | M | 52 | Bilateral orbital mass | 1 | 1% | 3 | Generalized | n | n | n | n | n | High |
| 12 | M | 31 | Nasal cavity | 13 | 32% | 2 | Limited | y | n | n | y | y | High |
| 13 | F | 16 | Nasal cavity | 1 | 7% | 1 | Limited | y | n | n | y | y | High |
| 14 | F | 47 | Orbital soft tissue | 24 | 53% | 2 | Generalized | y | y | n | y | y | High |
| 15 | F | 23 | Nasopharynx | 18 | 57% | 2 | Generalized | y | n | n | y | y | High |
| 16 | M | 44 | Nasal cavity | 0 | 0% | 2 | Generalized | y | n | n | y | y | High |
| 17*** | M | 36 | Nasal cavity | 43 | 47% | 1 | Generalized | y | n | n | y | y | Normal*** |
| 18*** | M | 36 | Eyelid/orbital fat | 53 | 81% | 2 | Generalized | y | n | n | y | y | *** |
| 19 | F | 64 | Nasal cavity | 8 | 13% | 2 | Generalized | y | y | n | n | n | High |
| 20 | F | 13 | Orbit | 15 | 30% | 1 | Generalized | y | y | n | n | n | Normal |
| 21 | F | 23 | Orbital mass | 69 | 83% | 3 | Generalized | y | n | n | y | y | Normal |
| 22 | F | 71 | Periorbital | 139 | 82% | 1 | Generalized | y | y | y | n | n | High |
| 23 | F | 31 | Nasal cavity | 55 | 73% | 1 | Generalized | y | n | n | y | y | High |
| 24 | F | 69 | Dura parietooccipital | 5 | 10% | 2 | Generalized | y | y | n | n | n | High |
| 25 | F | 40 | Pleura | 9 | 34% | 3 | Generalized | y | n | n | y | y | NA |
| 26 | F | 49 | Sinonasal | 37 | 44% | 1 | Limited | y | n | n | y | y | High |
*, **, *** denote the same patients. Dx: diagnosis; Path: pathology; ACR: American College of Rheumatology; ANCA: antineutrophil cytoplasmic antibody; PR3: proteinase 3; MPO: myeloperoxidase; NA: not available; CRP: c-reactive protein.